retroperitoneal sarcoma

1. Dr sumer yadavDr sumer yadav

2.  RETROPERITONEUM SPACERETROPERITONEUM SPACE

LARGE POTENTIAL SPACELARGE POTENTIAL SPACE
BOUNDED ANTERIORLY BY THEBOUNDED ANTERIORLY BY THE
POSTERIOR PERITONEUM.POSTERIOR PERITONEUM.

POSTERIORLY BY THE SPINE ANDPOSTERIORLY BY THE SPINE AND
BACK MUSCLESBACK MUSCLES

SUPERIORLY BY THE DIAPHRAGMSUPERIORLY BY THE DIAPHRAGM

INFERIORLY BY THE LEVATORSINFERIORLY BY THE LEVATORS

LATERALLY BY THE FLANKLATERALLY BY THE FLANK
MUSCLES AT THE LEVEL OF THEMUSCLES AT THE LEVEL OF THE
ANTERIOR SUPERIOR SPINE OFANTERIOR SUPERIOR SPINE OF
THE ILIAC CREST TO THE TIP OFTHE ILIAC CREST TO THE TIP OF
THE 12THE 12THTH
RIB.RIB.

3. INTRODUCTIONINTRODUCTION
 15% OF ALL SOFT TISSUE15% OF ALL SOFT TISSUE
SARCOMAS AND ONE THIRD OFSARCOMAS AND ONE THIRD OF
MALIGNANT RETROPERITONEALMALIGNANT RETROPERITONEAL
TUMORS.TUMORS.
MANAGEMENT CHALLENGEMANAGEMENT CHALLENGE
 BECAUSE OF THEIR FREQUENTBECAUSE OF THEIR FREQUENT
LATE PRESENTATIONLATE PRESENTATION
 LACK OF SPECIFIC SIGN &LACK OF SPECIFIC SIGN &
SYMPTOMSSYMPTOMS
 PROXIMITY TO VITAL STRUCTUREPROXIMITY TO VITAL STRUCTURE
 LARGE SIZELARGE SIZE

4. INTRODUCTION (Cont.)INTRODUCTION (Cont.)
SYNDROME ASSOCIATED WITH R.P.SYNDROME ASSOCIATED WITH R.P.
SARCOMA :SARCOMA :
 GARDNER SYN.GARDNER SYN.
 FAMILIAL RETINOBLASTOMAFAMILIAL RETINOBLASTOMA
 LI- FRAUMANI SYNLI- FRAUMANI SYN
 NEUROFIBROMATOSISNEUROFIBROMATOSIS
CARCINOGENS ASSOCIATED :CARCINOGENS ASSOCIATED :
 VINYL CHLORIDEVINYL CHLORIDE
 THORIUM DIOXIDETHORIUM DIOXIDE
 RADIATIONRADIATION

5. PRESENTATIONPRESENTATION
PATIENTS COMPLAIN WITHPATIENTS COMPLAIN WITH
ABDOMINAL MASSABDOMINAL MASS
BACK PAINBACK PAIN
WEIGHT LOSSWEIGHT LOSS
LOWER EXTREMITY SENSORYLOWER EXTREMITY SENSORY
CHANGESCHANGES
URINARY FREQUENCYURINARY FREQUENCY
INTESTINAL OBS.INTESTINAL OBS.

6. PRESENTATIONPRESENTATION
THE SIZE R.S. IS MORE THANTHE SIZE R.S. IS MORE THAN
10 cm. IN MOST REPORTED10 cm. IN MOST REPORTED
CASESCASES
EQUAL GENDEREQUAL GENDER
DISTRIBUTION.DISTRIBUTION.
THE MEAN AGE AT PRIMARYTHE MEAN AGE AT PRIMARY
PRESENTATION IS BETWEENPRESENTATION IS BETWEEN
49 AND 55 YEARS49 AND 55 YEARS

7. DISTRIBUTION OF HISTOLOGIC SUBTYPESDISTRIBUTION OF HISTOLOGIC SUBTYPES
OF RETROPERITONEAL SARCOMAOF RETROPERITONEAL SARCOMA
Liposarcoma
58%
Hemangiopericytoma
3%
Others
12%
MFH
4%
MPNST
3%
Leiomyosarcoma
20%
Liposarcoma Hemangiopericytoma Others MFH MPNST Leiomyosarcoma

8. HISTOLOGIC GRADEHISTOLOGIC GRADE
IS OF MORE PROGNOSTICIS OF MORE PROGNOSTIC
SIGNIFICANCE THAN THE CELLSIGNIFICANCE THAN THE CELL
OF ORIGIN.OF ORIGIN.
GRADING USES A COMPOSITEGRADING USES A COMPOSITE
OF HISTOPATHOLOGICOF HISTOPATHOLOGIC
FEATURES THAT INCLUDES :FEATURES THAT INCLUDES :

NEROSISNEROSIS

CELLULARITYCELLULARITY

PLEMORPHISMPLEMORPHISM

MITOSISMITOSIS

9. DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
 RETROPERITONEAL SARCOMARETROPERITONEAL SARCOMA
 LYMPHOMALYMPHOMA
 METASTATIC TESTICULAR CANCERMETASTATIC TESTICULAR CANCER
 ADRENAL TUMORSADRENAL TUMORS
 PANCREATIC TUMORSPANCREATIC TUMORS
 GASTROINTESTINAL STROMALGASTROINTESTINAL STROMAL
TUMORSTUMORS
 RENAL CELL CARCINOMARENAL CELL CARCINOMA
 RETROPERITONEAL FIBROSISRETROPERITONEAL FIBROSIS

10. DIAGNOSTIC EVALUATIONDIAGNOSTIC EVALUATION
CT SCANNINGCT SCANNING
 SIZE OF THE TUMORSIZE OF THE TUMOR
 ANY ANATOMIC CHANGESANY ANATOMIC CHANGES
SECONDARY TO ITS GROWTH ARESECONDARY TO ITS GROWTH ARE
EASILY VISUALIZEDEASILY VISUALIZED
 TUMOR INVASION OF ADJACENTTUMOR INVASION OF ADJACENT
ORGAN CAN ABE DEMONSTRATED ORORGAN CAN ABE DEMONSTRATED OR
SUGGESTEDSUGGESTED
 LYMPHOMA – HOMGENEOUS ANDLYMPHOMA – HOMGENEOUS AND
ENVELOPS THE IVC & AORTAENVELOPS THE IVC & AORTA
 SARCOMA – USUALLYSARCOMA – USUALLY
HETROGENEOUSHETROGENEOUS

12. DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)
THE FUNCTIONAL STATE OF ATTHE FUNCTIONAL STATE OF AT
LEAST ONE KIDNEY MUST BELEAST ONE KIDNEY MUST BE
DEMONSTRATED BY EITHER THEDEMONSTRATED BY EITHER THE
CONTRAST C.T. SCAN OR ANCONTRAST C.T. SCAN OR AN
EXCRETORY UROGRAM BECAUSEEXCRETORY UROGRAM BECAUSE
THE EN BLOC RESECTION OF ONETHE EN BLOC RESECTION OF ONE
KIDNEY IS OFTEN REQUIRED.KIDNEY IS OFTEN REQUIRED.
NEITHER C.T. SCAN NOR M.R.I. ISNEITHER C.T. SCAN NOR M.R.I. IS
SUPERIOR IN ASSESSMENT OFSUPERIOR IN ASSESSMENT OF
R.P. SARCOMA.R.P. SARCOMA.

13. DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)
ARTERIOGRAPHYARTERIOGRAPHY
 FINDING SUGGESTIVE OFFINDING SUGGESTIVE OF
NEOPLASIA INCLUDES :NEOPLASIA INCLUDES :
1.1. NEOVASCULARITYNEOVASCULARITY
2.2. TUMOR BLUSHTUMOR BLUSH
3.3. VESSEL ENCASEMENTVESSEL ENCASEMENT

14. DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)
 BECAUSE MALIGNANT FIBROUSBECAUSE MALIGNANT FIBROUS
HISTIOCYTOMA TENDS TO OCCUR INHISTIOCYTOMA TENDS TO OCCUR IN
THE RENAL AREA THETHE RENAL AREA THE
DEMONSTRATION OF AN EXTRADEMONSTRATION OF AN EXTRA
RENAL ARTERIAL SUPPLY ISRENAL ARTERIAL SUPPLY IS
HELPFUL IN DECIDING TO SAVE THEHELPFUL IN DECIDING TO SAVE THE
KIDNEY.KIDNEY.
 A DOMINANT LUMBER ORA DOMINANT LUMBER OR
INTERCOSTAL ARTERIAL SUPPLYINTERCOSTAL ARTERIAL SUPPLY
ADDS TO THE LIKELIHOOD THAT THEADDS TO THE LIKELIHOOD THAT THE
TUMOR HAS A RETROPERITONEALTUMOR HAS A RETROPERITONEAL
ORIGIN.ORIGIN.

15. INFERIORINFERIOR
VENAVENA
CAVAGRAMCAVAGRAM

16. DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)
PET SCANNINGPET SCANNING
 CURRENTLY NO DEFINED ROLECURRENTLY NO DEFINED ROLE
FOR POSITRON EMISSIONFOR POSITRON EMISSION
TOMOGRAPHY SCANNING INTOMOGRAPHY SCANNING IN
PRIMARY LEVELPRIMARY LEVEL
RETROPERITONEAL SARCOMA.RETROPERITONEAL SARCOMA.
 FLUORODEOXYGLUCOSE UPTAKEFLUORODEOXYGLUCOSE UPTAKE
DOES CORRELATE WITH TUMORDOES CORRELATE WITH TUMOR
GRADE IN SOFT TISSUE SARCOMAGRADE IN SOFT TISSUE SARCOMA
 NO DISCRIMINATING LOW-GRADENO DISCRIMINATING LOW-GRADE
TUMORS FROM BEING TUMORSTUMORS FROM BEING TUMORS

17. DIAGNOSTIC EVALUATION (Cont.)DIAGNOSTIC EVALUATION (Cont.)
FUTURE POTENTIAL USE :FUTURE POTENTIAL USE :
DETECTION METASTATICDETECTION METASTATIC
DISEASEDISEASE
DETECTION LOCALDETECTION LOCAL
RECURRENCERECURRENCE
DETECTION OF RESPONSEDETECTION OF RESPONSE
TO NEOADJUVANT THERAPYTO NEOADJUVANT THERAPY

18. BIOPSYBIOPSY
 HISTOLOGICAL DIAGNOSISHISTOLOGICAL DIAGNOSIS
SHOULD BE SECURED BY :SHOULD BE SECURED BY :
1.1. F.N.A.C.F.N.A.C.
2.2. TRU-CUT BIOPSYTRU-CUT BIOPSY
3.3. CT GUIDED CORE BIOPSYCT GUIDED CORE BIOPSY
 FOR SMALL MASSES THATFOR SMALL MASSES THAT
CAN BE RESECTED EN BLOCCAN BE RESECTED EN BLOC
PREOPERATIVE DIAGNOSISPREOPERATIVE DIAGNOSIS
LESS IMPORTANTLESS IMPORTANT

19. BIOPSY (Cont.)BIOPSY (Cont.)
 PRE-OPERATIVE BIOPSY IS FORPRE-OPERATIVE BIOPSY IS FOR
THOSE PATIENTS WHO ARE INVOLVEDTHOSE PATIENTS WHO ARE INVOLVED
IN NEOADJUVANT TREATMENTIN NEOADJUVANT TREATMENT
PROTOCOLS OR THOSE PATIENTS INPROTOCOLS OR THOSE PATIENTS IN
WHOM SYSTEMIC THERAPY WILL BEWHOM SYSTEMIC THERAPY WILL BE
PRIMARY TREATMENT MODALITYPRIMARY TREATMENT MODALITY
BECAUSE OF :BECAUSE OF :

THE PRESENCES OF METASTICTHE PRESENCES OF METASTIC
DISEASEDISEASE

LOCALLY ADVANCED DISEASELOCALLY ADVANCED DISEASE

DIAGNOSIS OF LYMPHOMADIAGNOSIS OF LYMPHOMA

20. STAGING SOFT TISSUE SARCOMASTAGING SOFT TISSUE SARCOMA
T1T1 TUMOR < 5 cmTUMOR < 5 cm
T1aT1a SUPERFICIAL TUMORSUPERFICIAL TUMOR
T1bT1b DEEP TUMORDEEP TUMOR
T2T2 TUMOR > 5 cm INTUMOR > 5 cm IN
GREATEST DIMENSIONGREATEST DIMENSION
T2aT2a SUPERFICIAL TUMORSUPERFICIAL TUMOR
T2bT2b DEEP TUMORDEEP TUMOR

21. STAGING SOFT TISSUE SARCOMA (Cont.)STAGING SOFT TISSUE SARCOMA (Cont.)
REGIONAL NODES (N)REGIONAL NODES (N)
 NXNX REGIONAL LYMPH NODESREGIONAL LYMPH NODES
CANNOT BE ASSESSEDCANNOT BE ASSESSED
 N0N0 NO REGIONAL LYMPH NODENO REGIONAL LYMPH NODE
METASTASISMETASTASIS
 N1N1 REGIONAL LYMPH NODEREGIONAL LYMPH NODE
METASTASISMETASTASIS
DISTANT METASTASES (M)DISTANT METASTASES (M)
 MXMX DISTANT METASTASISDISTANT METASTASIS
CANNOTCANNOT BE ASSESSEDBE ASSESSED
 M0M0 NO DISTANT METASTASISNO DISTANT METASTASIS
 M1M1 DISTANT METASTASISDISTANT METASTASIS

22. STAGING SOFT TISSUE SARCOMASTAGING SOFT TISSUE SARCOMA
(Cont.)(Cont.)
Stage Grouping
Stage I
A (LOW GRADE, SMALL, SUPERFICIAL, DEEP) G1-2 T1a-b N0 M0
B (LOW GRADE, LARGE, SUPERFICIAL) G1-2 T2a N0 M0
STAGE II
A (LOW GRADE LARGE, DEEP) G1-2 T2b N0 M0
B (HIGH GRADE, SMALL, SUPERFICIAL, DEEP) G3-4 T1a-b N0 M0
C (HIGH GRADE, LARGE SUPERFICIAL) G3-4 T2a N0 M0
STAGE III
HIGH GRADE, LARGE, DEEP G3-4 T2b N0 M0
STAGE IV
ANY MATASTASIS ANY G ANY T N1 M0
ANY G ANY T N0 M1

23. ALGORITHM FOR MANAGEMENT OFALGORITHM FOR MANAGEMENT OF
RETROPERITONEAL SARCOMASRETROPERITONEAL SARCOMAS
PRIMARY RESECTABLE
RETROPERITONEAL SARCOMA
BIOPSY
NEOADJUVANT TRIAL
RESECTION
FOLLOW
CLINICALLY
RESECTION
FOLLOW
CLINICALLY

24. MANAGEMENT OF LOCALLY ADVANCED &MANAGEMENT OF LOCALLY ADVANCED &
METASTATIC DISEASEDISEASE
LOCALLY ADVANCED & METASTATIC
RETROPERITONEAL SARCOMA
ASYMPTOMATIC
CLINICAL
OBSERVATION
± CHEMOTHERAPY
± INVESTIGATION AGENTS
± RADIATION THERAPY
MECHANICAL SYMPTOMS
PALLIATIVE RESECTION
± CHEMOTHERAPY
± INVESTIGATION AGENTS
± RADIATION THERAPY
CLINICAL
OBSERVATION

25. SURGICAL RESECTIONSURGICAL RESECTION
 REMAINS THE ONLY POTENTIALLYREMAINS THE ONLY POTENTIALLY
CURATIVE MODALITY IN PATIENTSCURATIVE MODALITY IN PATIENTS
WIT RETROPERITONEAL SARCOMAWIT RETROPERITONEAL SARCOMA
 PRIMARY NONMETASTATICPRIMARY NONMETASTATIC
RETROPERITONEAL SARCOMARETROPERITONEAL SARCOMA
RESECTABILITY RATES HAVERESECTABILITY RATES HAVE
RANGED FROM 59% TO 95%.RANGED FROM 59% TO 95%.
 RESECTABILITY RATES NOTRESECTABILITY RATES NOT
SIGNIFICANTLY ASSOCIATED WITHSIGNIFICANTLY ASSOCIATED WITH
TUMOR SIZE , GRADE ORTUMOR SIZE , GRADE OR
HISTOLOGIC TYPE.HISTOLOGIC TYPE.

26. SURGICAL RESECTIONSURGICAL RESECTION (Cont.)(Cont.)
 THE MOST COMMON ORGANTHE MOST COMMON ORGAN
REQUIRING SIMULTANEOUS ENREQUIRING SIMULTANEOUS EN
BLOC RESECTION ARE KIDNEY.BLOC RESECTION ARE KIDNEY.
ADRENAL, COLON, PANCREAS ANDADRENAL, COLON, PANCREAS AND
SPLEENSPLEEN
 REASONS FOR UNRESECTABILITYREASONS FOR UNRESECTABILITY
OR INCOMPLETE RESECTION ATOR INCOMPLETE RESECTION AT
THE TIME OF EXPORATION INCLUDETHE TIME OF EXPORATION INCLUDE
VASCULAR INVOLVEMENTVASCULAR INVOLVEMENT
PERITONEAL, METASTASIS ANDPERITONEAL, METASTASIS AND
MULTIFOCALITYMULTIFOCALITY

27. OPERATIVE CONSIDERATIONSOPERATIVE CONSIDERATIONS
ALL PATIENTS SHOULDALL PATIENTS SHOULD
UNDERGO A FULL BOWELUNDERGO A FULL BOWEL
PREPARATION BECAUSEPREPARATION BECAUSE
A LIMITED RESECTION OFA LIMITED RESECTION OF
THE COLON OR RECTUMTHE COLON OR RECTUM
IS COMMONLY REQUIREDIS COMMONLY REQUIRED

28. OPERATIVE CONSIDERATIONSOPERATIVE CONSIDERATIONS
MIDLINE INCISION IS USUALLYMIDLINE INCISION IS USUALLY
BEST FOR THE INITIALBEST FOR THE INITIAL
EXPLORATIONEXPLORATION
IF THE TUMOR IS IN THEIF THE TUMOR IS IN THE
UPPER RETROPERITONEUMUPPER RETROPERITONEUM
TOWARDS OR INVADING THETOWARDS OR INVADING THE
DIAPHRAGM, ADIAPHRAGM, A
THORACOABDOMINALTHORACOABDOMINAL
APPROACH MAY BEAPPROACH MAY BE
INDICATEDINDICATED

29. OPERATIVE CONSIDERATIONS (Conts.)OPERATIVE CONSIDERATIONS (Conts.)
THE ABDOMINAL PORTIONTHE ABDOMINAL PORTION
OF THE INCISIONS ISOF THE INCISIONS IS
OPENED FIRST FOR THEOPENED FIRST FOR THE
EXPLORATION TOEXPLORATION TO
DETERMINEDETERMINE
RESECTABILITY AND ARESECTABILITY AND A
CAREFUL SEARCH IS MADECAREFUL SEARCH IS MADE
FOR HEPATIC ORFOR HEPATIC OR
PERITONEAL MATASTASES.PERITONEAL MATASTASES.

30. OPERATIVE CONSIDERATIONS (Conts.)OPERATIVE CONSIDERATIONS (Conts.)
THE FLANK APPROACH ISTHE FLANK APPROACH IS
LESS SATISFACTORY THANLESS SATISFACTORY THAN
AN ABDOMINAL INCISION INAN ABDOMINAL INCISION IN
ALLOWING THE SURGEONALLOWING THE SURGEON
TO PERFORM AN EN BLOCTO PERFORM AN EN BLOC
RESECTION OF INVOLVEDRESECTION OF INVOLVED
ORGANS OR TO CONTROLORGANS OR TO CONTROL
THE MAJOR ARTERIES ANDTHE MAJOR ARTERIES AND
VEINS SUPPLYING THEVEINS SUPPLYING THE
TUMORTUMOR

31. OPERATIVE CONSIDERATIONS (Cont.)OPERATIVE CONSIDERATIONS (Cont.)
INCISIONAL WEDGE BIOPSIESINCISIONAL WEDGE BIOPSIES
SHOULD BE OBTAINED ONLYSHOULD BE OBTAINED ONLY
FROM PATIENTS WHO HAVEFROM PATIENTS WHO HAVE
OBVIOUSLY INOPERABLEOBVIOUSLY INOPERABLE
DISEASE OR WHERE LYMPHOMADISEASE OR WHERE LYMPHOMA
IS SUSPECTEDIS SUSPECTED
GREAT CARE MUST BE TAKEN TOGREAT CARE MUST BE TAKEN TO
ISOLATE THE AREA OF BIOPSYISOLATE THE AREA OF BIOPSY
AND TO OBTAIN ABSOLUTEAND TO OBTAIN ABSOLUTE
HEMOSTASISHEMOSTASIS

32. OPERATIVE CONSIDERATIONS (Cont.)OPERATIVE CONSIDERATIONS (Cont.)
LOCALIZED TUMOR :LOCALIZED TUMOR :

THIS SHOULD BETHIS SHOULD BE
REMOVED EN BLOC WHICHREMOVED EN BLOC WHICH
MAY INCLUDE AN EN BLOCMAY INCLUDE AN EN BLOC
RESECTION OF INVOLVEDRESECTION OF INVOLVED
SURROUNDING ORGAN.SURROUNDING ORGAN.

THERE SHOULD BE 1 TO 2THERE SHOULD BE 1 TO 2
cm OF NORMAL MARGIN.cm OF NORMAL MARGIN.

33. OPERATIVE CONSIDERATIONS (Cont.)OPERATIVE CONSIDERATIONS (Cont.)

TUMOR SHOULD BETUMOR SHOULD BE
REMOVED ALONG WITHREMOVED ALONG WITH
THIS PSEUDOCAPSULETHIS PSEUDOCAPSULE

FIXATION IS NOT A SIGN OFFIXATION IS NOT A SIGN OF
UNRESECTABILITY UNLESSUNRESECTABILITY UNLESS
THERE IS EXTENSIVETHERE IS EXTENSIVE
INVOLVEMENT OFINVOLVEMENT OF
IRREPLACEABLE ORIRREPLACEABLE OR
UNREMOVABLE STRUCTURESUNREMOVABLE STRUCTURES

34. AA
SEGMENTSEGMENT
OF SMALLOF SMALL
BOWELBOWEL
ANDAND
COLONCOLON
ADHERENTADHERENT
TO THETO THE
LEFTLEFT
RETROPERRETROPER
- ITONEAL- ITONEAL
SARCOMASARCOMA

35. MANAGEMENT OF THE KIDNEYMANAGEMENT OF THE KIDNEY
 NEPHRECTOMY IS FREQUENTLYNEPHRECTOMY IS FREQUENTLY
PERFORMED AT THE TIME OFPERFORMED AT THE TIME OF
RESECTION OF LARGERESECTION OF LARGE
RETROPERITONEAL SARCOMAS.RETROPERITONEAL SARCOMAS.
 DIRECT RENAL, RENAL CAPSULE ORDIRECT RENAL, RENAL CAPSULE OR
RENAL VASCULAR INVASION BYRENAL VASCULAR INVASION BY
TUMOR OCCURS IN LESS THAN 30%TUMOR OCCURS IN LESS THAN 30%
 MORE COMMONLY IN 70% OF CASES,MORE COMMONLY IN 70% OF CASES,
THE TUMOR WILL ENCASE OR BETHE TUMOR WILL ENCASE OR BE
ADHERENT TO THE KIDNEY WITHOUTADHERENT TO THE KIDNEY WITHOUT
HISTOLOGICAL INVASION.HISTOLOGICAL INVASION.

36. MANAGEMENT OF THEMANAGEMENT OF THE
INFERIOR VENA CAVAINFERIOR VENA CAVA
RESECTION OF THE INFERIORRESECTION OF THE INFERIOR
VENA CAVA SHOULD BEVENA CAVA SHOULD BE
UNDERTAKEN IN SELECTEDUNDERTAKEN IN SELECTED
PATIENTS WHEN COMPLETEPATIENTS WHEN COMPLETE
GROSS RESECTION OFGROSS RESECTION OF
TUMOR IS LIMITED BYTUMOR IS LIMITED BY
INVOLVEMENT OF THEINVOLVEMENT OF THE
INFERIOR VENA CAVA.INFERIOR VENA CAVA.

37. REPAIR OFREPAIR OF
INFERIOR VENA CAVAINFERIOR VENA CAVA
PRIMARY REPAIRPRIMARY REPAIR
AUTOLOGOUS PATCH REPAIRAUTOLOGOUS PATCH REPAIR
AUTOLOGOUS VEIN REPAIRAUTOLOGOUS VEIN REPAIR
PROSTHETIC TUBE GRAFTINGPROSTHETIC TUBE GRAFTING
LIGATION OF INFERIOR VENALIGATION OF INFERIOR VENA
CAVACAVA

38. ROLE OF INCOMPLETE RESECTIONROLE OF INCOMPLETE RESECTION
INCOMPLETE GROSSINCOMPLETE GROSS
RESECTION OR DEBULKING ISRESECTION OR DEBULKING IS
NOT ADVOCATED BECAUSE ITNOT ADVOCATED BECAUSE IT
HAS NOT BEEN ASSOCIATEDHAS NOT BEEN ASSOCIATED
WITH IMPROVED SURVIVAL.WITH IMPROVED SURVIVAL.
DELIBERATE PARTIALDELIBERATE PARTIAL
RESECTION OF MOSTRESECTION OF MOST
RETROPERITONEAL SARCOMARETROPERITONEAL SARCOMA
SHOULD LIMITED TO RELIEF OFSHOULD LIMITED TO RELIEF OF
INTESTINAL OBSTRUCTIONINTESTINAL OBSTRUCTION

39. RADIATION THERAPYRADIATION THERAPY
TREATMENT OPTIONSTREATMENT OPTIONS
E.B.R.T.E.B.R.T.

PREOPERATIVEPREOPERATIVE

POSTOPERATIVEPOSTOPERATIVE
I.O.R.T. (10 – 15 Gy)I.O.R.T. (10 – 15 Gy)
BRACHYTHERAPYBRACHYTHERAPY
E.B.R.T. + I.O.R.T. ORE.B.R.T. + I.O.R.T. OR
BRACHYTHERAPY MOREBRACHYTHERAPY MORE
EFFECTIVEEFFECTIVE

40. RADIATION THERAPY (Cont.)RADIATION THERAPY (Cont.)
THE HIGH DOSE REQUIREDTHE HIGH DOSE REQUIRED
AROUND 60 Gy.AROUND 60 Gy.
EBRT HAVE LIMITED ROLEEBRT HAVE LIMITED ROLE
BECAUSE OF LOW TOXICITYBECAUSE OF LOW TOXICITY
THRESHOLD OF SURROUNDINGTHRESHOLD OF SURROUNDING
TISSUE.TISSUE.
EBRT ASSOCIATED WITH DELAY INEBRT ASSOCIATED WITH DELAY IN
TIME OF LOCAL RECURRENCETIME OF LOCAL RECURRENCE
BUT NO IMPROVEMENT INBUT NO IMPROVEMENT IN
SURVIVAL.SURVIVAL.

41. CHEMOTHERAPYCHEMOTHERAPY
 NO PROVEN ROLE FOR ADJUVANTNO PROVEN ROLE FOR ADJUVANT
CHEMOTHERAPY IN COMPLETELYCHEMOTHERAPY IN COMPLETELY
RESECTED R.P. SARCOMA.RESECTED R.P. SARCOMA.
 CHEMOTHERAPY MAY BE USED IN :CHEMOTHERAPY MAY BE USED IN :
1.1. LOCALLY UNRESECTABLE DISEASELOCALLY UNRESECTABLE DISEASE
2.2. METASTIC R.P. SARCOMAMETASTIC R.P. SARCOMA
3.3. PATIENT UNDERGOESPATIENT UNDERGOES
NEOADJUVANT TRAILNEOADJUVANT TRAIL

42. MANAGEMENT OF LOCALMANAGEMENT OF LOCAL
RECURRENCERECURRENCE
LOCAL RECURRENCE OCCUR INLOCAL RECURRENCE OCCUR IN
41% OF PATIENTS IN FIVE YEARS41% OF PATIENTS IN FIVE YEARS
LOCAL RECURRENCE IS PRIMARYLOCAL RECURRENCE IS PRIMARY
CAUSE OF DISEASE SPECIFICCAUSE OF DISEASE SPECIFIC
MORTALITY.MORTALITY.
COMPLETE SURGICAL RESECTIONCOMPLETE SURGICAL RESECTION
IS MOST EFFECTIVE THERAPYIS MOST EFFECTIVE THERAPY
FOR LOCAL RECURRENCEFOR LOCAL RECURRENCE

43. WHEN TO OPERATE?WHEN TO OPERATE?
PATIENTS WITH FIRSTPATIENTS WITH FIRST
LOCAL RECURRENCELOCAL RECURRENCE
SHOULD BE CONSIDER FORSHOULD BE CONSIDER FOR
REEXPLORATION.REEXPLORATION.
COMPLETE RESECTABILITYCOMPLETE RESECTABILITY
RATE AFTER FIRSTRATE AFTER FIRST
RECURRENCE IS 54 – 82%.RECURRENCE IS 54 – 82%.

44. WHEN TO OPERATE?WHEN TO OPERATE? (Cont.)(Cont.)
IN PATIENTS WITH SHORTIN PATIENTS WITH SHORT
DISEASE FREE INTERVAL ADISEASE FREE INTERVAL A
PERIOD OF OBSERVATIONPERIOD OF OBSERVATION
SHOULD BE FOLLOWEDSHOULD BE FOLLOWED
BEFORE OPERATION TOBEFORE OPERATION TO
EXCLUDE THEEXCLUDE THE
DEVELOPMENT OFDEVELOPMENT OF
DISSEMINATED DIS.DISSEMINATED DIS.

45. DISTANT METASTASISDISTANT METASTASIS
MOST COMMON SITE FORMOST COMMON SITE FOR
DISTANT METASTASIS ISDISTANT METASTASIS IS
LUNG & LIVER.LUNG & LIVER.
RESECTION OF DISTANTRESECTION OF DISTANT
METASTASIS RESECTED TOMETASTASIS RESECTED TO
THE PATIENTS IN WHOM ATHE PATIENTS IN WHOM A
COMPLETE RESECTION CANCOMPLETE RESECTION CAN
BE PERFORMED.BE PERFORMED.

46. SURVIVAL & PREDICTORS OFSURVIVAL & PREDICTORS OF
OUTCOMEOUTCOME
 FACTOR ASSOCIATED WITH POORFACTOR ASSOCIATED WITH POOR
SURVIVAL.SURVIVAL.
1.1. INCOMPLETE GROSS RESECTIONINCOMPLETE GROSS RESECTION
2.2. UNRESECTABILITYUNRESECTABILITY
3.3. HIGH GRADEHIGH GRADE
 FACTOR ASSOCIATED WITH LOCALFACTOR ASSOCIATED WITH LOCAL
RECURRENCERECURRENCE
1.1. HIGH GRADEHIGH GRADE
2.2. LIPOSARCOMA HISTOLOGYLIPOSARCOMA HISTOLOGY

47. SURVIVAL & PREDICTORS OFSURVIVAL & PREDICTORS OF
OUTCOMEOUTCOME (Cont.)(Cont.)
 FACTOR ASSOCIATED WITHFACTOR ASSOCIATED WITH
DISTANT METASTASIS :-DISTANT METASTASIS :-
1.1. INCOMPLETE RESECTIONINCOMPLETE RESECTION
2.2. HIGH GRADEHIGH GRADE
 LIPOSARCOMA ASSOCIATEDLIPOSARCOMA ASSOCIATED
WITH REDUCED RISK OFWITH REDUCED RISK OF
DISTANT MATASTASISDISTANT MATASTASIS

48. FOLLOW - UPFOLLOW - UP
 GOAL OF FOLLOW – UP IS TO DETECTGOAL OF FOLLOW – UP IS TO DETECT
CURABLE RECURRENT ORCURABLE RECURRENT OR
METASTATIC DISEASE.METASTATIC DISEASE.
 PATIENTS ARE EVALUATEDPATIENTS ARE EVALUATED
CLINICALLY EVERY 4 MONTHS FOR 3CLINICALLY EVERY 4 MONTHS FOR 3
YEARS AND EVERY 6 MONTHS THEREYEARS AND EVERY 6 MONTHS THERE
AFTER.AFTER.
 CT SCAN ARE PERFORMED INCT SCAN ARE PERFORMED IN
PATIENTS IN WHOM OPERATION ISPATIENTS IN WHOM OPERATION IS
CONSIDERED AT 6-12 MONTHCONSIDERED AT 6-12 MONTH
INTERVALINTERVAL

49. CONCLUSIONCONCLUSION
RETROPERITONEAL SARCOMARETROPERITONEAL SARCOMA
ARE RARE.ARE RARE.
THEY USUALLY REACH A LARGETHEY USUALLY REACH A LARGE
SIZE BEFORE PRESENTATION.SIZE BEFORE PRESENTATION.
LIPOSARCOMAS IS MOSTLIPOSARCOMAS IS MOST
COMMON.COMMON.
CT SCAN IS THE MOSTCT SCAN IS THE MOST
IMPORTANT IN PLANNINGIMPORTANT IN PLANNING
RESECTION.RESECTION.

50. CONCLUSIONCONCLUSION (Cont.)(Cont.)
AN ABDOMINAL APPROACH ASAN ABDOMINAL APPROACH AS
USUALLY ADVISEDUSUALLY ADVISED
CURABLE LESION SHOULD BECURABLE LESION SHOULD BE
REMOVED RADICALLY AND NOTREMOVED RADICALLY AND NOT
REMOVED FROM THEIRREMOVED FROM THEIR
PSEUDOCAPSULEPSEUDOCAPSULE
50% OF TUMOR ARE50% OF TUMOR ARE
RESECTABLE AND 75% REQUIRERESECTABLE AND 75% REQUIRE
RESECTION OF ADJACENTRESECTION OF ADJACENT
ORGANS.ORGANS.

51. CONCLUSIONCONCLUSION (Cont.)(Cont.)
SURVIVAL DEPENDENT UPONSURVIVAL DEPENDENT UPON
GRADE OF TUMOR ANDGRADE OF TUMOR AND
STAGE.STAGE.
80% OF PATIENTS SUFFER80% OF PATIENTS SUFFER
RECURRENCE.RECURRENCE.
ADJUVANT CHEMOTHERAPYADJUVANT CHEMOTHERAPY
HAS NO ROLE OUTSIDEHAS NO ROLE OUTSIDE
CLINICAL TRIALCLINICAL TRIAL

52. CONCLUSIONCONCLUSION (Cont.)(Cont.)
RADIATION: NO PROVEN BENEFITRADIATION: NO PROVEN BENEFIT
BUT DATA SUGGESTS THATBUT DATA SUGGESTS THAT
LOCAL CONTROL IS IMPROVEDLOCAL CONTROL IS IMPROVED
WITH RADIATION.WITH RADIATION.
THERE IS A PROBLEM OF DOSE-THERE IS A PROBLEM OF DOSE-
RELATED TOXICITY.RELATED TOXICITY.
COMBINED EXTERNAL BEAMCOMBINED EXTERNAL BEAM
RADIATION THERAPY AND BOOSTRADIATION THERAPY AND BOOST
APPEARS TO BE SUPERIOR FORAPPEARS TO BE SUPERIOR FOR
RESPONSE.RESPONSE.